ECM Flashcards

1
Q

What are the two main types of ECM components?

A

Fibres - I.e collagen and elastin

Ground substance - I.e proteoglycans, glycosaminoglycans, glycoproteins

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2
Q

Give an example of a calcified ECM? How does this aid function?

A

Bone

Gives bone mechanical properties

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3
Q

Describe the structure of collagen.

A

Triple helix

GLY-X-Y repeating

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4
Q

What are the two main types of collagen?

A

Fibrillar - I.e type 1 & 2

Sheet forming - I.e type 4

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5
Q

Give examples of each type of collagen.

A
1 - bone, tendon, ligaments, dermis
2 - hyaline cartilage 
3 - liver, lymphoid organs, bone marrow 
4 - basement membrane 
4 - linker to basement membrane, cornea
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6
Q

What glycoprotein is required to arrange elastin into fibres?

A

Fibrillin

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7
Q

Where in the body is elastin found?

A

Skin, lungs, blood vessels

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8
Q

What is the structure of a proteoglycan?

A

Carbohydrate GAGs arranged around a core protein in a bottle brush structure

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9
Q

Name different GAGs found in different tissues

A

Chondroitin sulphate
Keratin sulphate - cartilage

Hyaluronic acid - synovial fluid

Heparan sulphate - basement membrane

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10
Q

Name and describe the structure of different proteoglycans in different tissues.

A

Aggrecan - chondroitin sulphate and keratin sulphate - cartilage

Perlecan - heparan sulphate - basement membrane

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11
Q

What is the function of the glycoprotein fibrillin?

A

Controls deposition and orientation of elastin

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12
Q

What is the function of glycoprotein fibronectin?

A

Linker role in basement membrane

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13
Q

Describe how collagen is synthesised.

A

Synthesised as protocollagen

PTMs - glycosylation, hydroxylation

Assembly - triple helix

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14
Q

Describe how elastin is synthesised?

A

Synthesised as tropoelastin

PTMs - hydroxylation

Assembly - fibrillin scaffold cross liked fibres

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15
Q

Describe the formation of proteoglycans.

A

Core protein synthesised in RER

GAGs added in Golgi

Secreted outside cell by exocytosis

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16
Q

What is the basement membrane in epithelium composed of?

A

Collagen, laminin, perlecan, enactin and fibronectin

17
Q

What are the 3 layers of the basement membrane?

A

Lamina Lucida, lamina densa, lamina fibroreticularis

18
Q

What are the functions of the basement membrane?

A

Support, binding to underlying connective tissue, mediates signals between cells and connective tissue, permeable to nutrients, path for cell migration

19
Q

What does problems does defective elastin give?

A

Arterial defects

20
Q

What problems does defective fibrillin-1 give?

A

Marfan syndrome

21
Q

What problem does defective collagen give?

A

Ehlers-danlos syndrome